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Kernicterus

Definition

Kernicterus is a rare neurological condition that occurs in some newborns with severe jaundice.

Alternative Names

Bilirubin-induced neurologic dysfunction (BIND); Bilirubin encephalopathy (BE)

Causes

Kernicterus is caused by very high levels of bilirubin. Bilirubin is a yellow pigment that is created as the body gets rid of old red blood cells. High levels of bilirubin in the body can cause the skin to look yellow ( jaundice).

If levels of bilirubin are very high or a baby is very ill, the substance will move out of the blood and collect in the brain tissue. This can lead to serious complications, including brain damage and hearing loss. The term "kernicterus" refers to the yellow staining caused by bilirubin. This is seen in parts of the brain on autopsy.

Kernicterus most often develops in the first week of life. However, but may be seen up until the third week. Some newborns with Rh hemolytic disease are at high risk for severe jaundice that can lead to this condition. Rarely, kernicterus can develop in seemingly healthy babies.

Symptoms

The symptoms depend on the stage of kernicterus.

Early stage:

  • Extreme jaundice
  • Absent startle reflex
  • Poor feeding or sucking
  • Extreme sleepiness (lethargy) and low muscle tone (hypotonia)

Mid stage:

  • High-pitched cry
  • Arched back with neck hyperextended backwards (high muscle tone/hypertonia)
  • Bulging fontanel (soft spot)
  • Seizures

Late stage:

  • High-frequency hearing loss
  • Intellectual disability
  • Muscle rigidity
  • Speech difficulties
  • Seizures
  • Movement disorder

Exams and Tests

A blood test will show a high bilirubin level (greater than 20-25 mg/dL).

Note: Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.

Treatment

Treatment depends on how old the baby is (in hours) and whether the baby has any risk factors (such as prematurity). It may include:

  • Light therapy (phototherapy)
  • Exchange transfusions

Outlook (Prognosis)

Kernicterus is a serious condition. Many infants with late-stage nervous system complications die.

Possible Complications

  • Permanent brain damage
  • Hearing loss
  • Death

When to Contact a Medical Professional

Get medical help right away if your baby has signs of this condition.

Prevention

Treating jaundice or conditions that may lead to it can help prevent kernicterus. Infants with the first signs of jaundice have bilirubin level measured within 24 hours. If the level is high, the infant should be screened for diseases that involve the destruction of red blood cells (hemolysis).

All newborns have a follow-up appointment within 2 to 3 days after leaving the hospital. This is very important for late preterm or early term babies (born more than 2-3 weeks before their due date).

References

American Academy of Pediatrics. Committee on Fetus and Newborn. Hospital stay for healthy term newborns. Pediatrics. 2010; 125: 405.

American Academy of Pediatrics, Subcommittee on Hyperbilirubinemia Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114: 297-316.

Johnson L, Bhutani V. The clinical syndrome of bilirubin-induced neurologic dysfunction. Seminars in Perinatology. 2011; 35(3):101-113.


Review Date: 5/7/2013
Reviewed By: Kimberly G Lee, MD, MSc, IBCLC, Associate Professor of Pediatrics, Division of Neonatology, Medical University of South Carolina, Charleston, SC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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